Choledochal Cysts: In Brief with Dr. Alexander Bondoc

Abstract

Choledochal cysts are a core pathology in pediatric surgery, affecting 1/100,000 live births in the western world, and 1/13,000 in eastern asia. These cysts are classified by the Todani classification, types I-V, in respect to their location and underlying pathophysiology. Infants and children presenting with stigmata of biliary disease should undergo evaluation for choledocal cyst. Workup includes axial imaging, ultrasonography, and laboratory investigation. A liver biopsy is necessary in neonates and newborns to rule out cystic biliary atresia, which would require further evaluation and management. Large choledochal cysts may be visualized on prenatal ultrasound, and warrant referral to a fetal care center for postnatal work up and monitoring. Management of choledochal cysts is dependent on the anatomic variant and spans from ERCP with sphincterotomy, to cyst excision with ductal and alimentary tract reconstruction. Type V choledochal cysts may require liver transplantation. Long term follow up is required due to an enhanced risk of future malignancy, even after resection.